BACKGROUND: Lung function is an important indicator of cystic fibrosis disease status and those with better forced expiratory volume in 1 s (FEV(1))% predicted have tended to report a better health-related quality of life (HRQoL) in cross-sectional studies. The relationship between lung function and HRQoL over time is unknown. This work assesses the natural progression of HRQoL reporting over many years and compares assessments across a whole decade and evaluates the relationship between lung function and HRQoL longitudinally. METHODS: Demographic (age, gender), clinical (FEV(1)% predicted, body mass index, diabetes, Burkholderia cepacia complex, intravenous access device and nutritional status) and HRQoL (Cystic Fibrosis Quality of Life Questionnaire) variables were obtained every 2 years over a 12-year period (seven time points from 1998 to 2010). RESULTS: HRQoL and lung function declined slowly over time and significant decade changes were observed for FEV(1)% predicted and the nine domains of the Cystic Fibrosis Quality of Life Questionnaire. The results of random coefficient modelling indicated that, at the population level, decreasing FEV(1)% predicted was associated with decreasing HRQoL after adjusting for confounding variables. However, the percentage of patients for whom a decrease in lung function was associated with a decrease in HRQoL differed according to the quality of life domain. CONCLUSIONS: HRQoL and FEV(1)% predicted decline slowly; nevertheless, a decrease in lung function predicted a decrease in HRQoL over time.
BACKGROUND: Lung function is an important indicator of cystic fibrosis disease status and those with better forced expiratory volume in 1 s (FEV(1))% predicted have tended to report a better health-related quality of life (HRQoL) in cross-sectional studies. The relationship between lung function and HRQoL over time is unknown. This work assesses the natural progression of HRQoL reporting over many years and compares assessments across a whole decade and evaluates the relationship between lung function and HRQoL longitudinally. METHODS: Demographic (age, gender), clinical (FEV(1)% predicted, body mass index, diabetes, Burkholderia cepacia complex, intravenous access device and nutritional status) and HRQoL (Cystic Fibrosis Quality of Life Questionnaire) variables were obtained every 2 years over a 12-year period (seven time points from 1998 to 2010). RESULTS: HRQoL and lung function declined slowly over time and significant decade changes were observed for FEV(1)% predicted and the nine domains of the Cystic Fibrosis Quality of Life Questionnaire. The results of random coefficient modelling indicated that, at the population level, decreasing FEV(1)% predicted was associated with decreasing HRQoL after adjusting for confounding variables. However, the percentage of patients for whom a decrease in lung function was associated with a decrease in HRQoL differed according to the quality of life domain. CONCLUSIONS: HRQoL and FEV(1)% predicted decline slowly; nevertheless, a decrease in lung function predicted a decrease in HRQoL over time.
Authors: Karine Chevreul; Morgane Michel; Karen Berg Brigham; Julio López-Bastida; Renata Linertová; Juan Oliva-Moreno; Pedro Serrano-Aguilar; Manuel Posada-de-la-Paz; Domenica Taruscio; Arrigo Schieppati; Georgi Iskrov; Márta Péntek; Johann Matthias Graf von der Schulenburg; Panos Kanavos; Ulf Persson; Giovani Fattore Journal: Eur J Health Econ Date: 2016-04-08
Authors: Alyssa A Perez; Steven R Hays; Allison Soong; Ying Gao; John R Greenland; Lorriana E Leard; Rupal J Shah; Jeffrey Golden; Jasleen Kukreja; Aida Venado; Mary Ellen Kleinhenz; Jonathan P Singer Journal: Pediatr Pulmonol Date: 2020-04-01
Authors: Christopher McNamara; Mahrya Johnson; Lisa Read; Heidi Vander Velden; Megan Thygeson; Meixia Liu; Laura Gandrud; John McNamara Journal: Evid Based Complement Alternat Med Date: 2016-12-19 Impact factor: 2.629
Authors: Jennifer Ruddy; Julia Emerson; Sharon McNamara; Alan Genatossio; Cora Breuner; Tracy Weber; Margaret Rosenfeld Journal: Glob Adv Health Med Date: 2015-11-01
Authors: A Shahabeddin Parizi; P F M Krabbe; E A M Verschuuren; R A S Hoek; J M Kwakkel-van Erp; M E Erasmus; W van der Bij; K M Vermeulen Journal: Am J Transplant Date: 2017-10-12 Impact factor: 8.086